Effects of mutations in mitochondrial cytochrome b in yeast and man. Deficiency, compensation and disease

Eur J Biochem. 2001 Mar;268(5):1155-62. doi: 10.1046/j.1432-1327.2001.02010.x.


The mitochondrial cytochrome bc(1) complex is a key protonmotive component of eukaryotic respiratory chains. The mitochondrially encoded cytochrome b forms, with cytochrome c(1) and the iron--sulfur protein, the catalytic core of this multimeric enzyme. Mutations of cytochrome b have been reported in association with human diseases. In the highly homologous yeast cytochrome b, several mutations that impair the respiratory function, and reversions that correct the defect, have been described. In this paper, we re-examine the mutations in the light of the atomic structure of the complex, and discuss the possible effect, at enzyme level, of the human cytochrome b mutations and the correcting effect of the reversions.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Cardiomyopathies / enzymology*
  • Cardiomyopathies / genetics
  • Cardiomyopathies / metabolism
  • Cell Respiration
  • Cytochrome b Group / chemistry
  • Cytochrome b Group / deficiency*
  • Cytochrome b Group / genetics
  • Cytochrome b Group / metabolism*
  • Electron Transport Complex III / chemistry
  • Electron Transport Complex III / genetics
  • Electron Transport Complex III / metabolism
  • Humans
  • Mitochondria / enzymology*
  • Mitochondria / genetics
  • Mitochondria / metabolism
  • Mitochondrial Myopathies / enzymology*
  • Mitochondrial Myopathies / genetics
  • Mitochondrial Myopathies / metabolism
  • Models, Molecular
  • Mutation / genetics*
  • Suppression, Genetic / genetics
  • Yeasts / enzymology*
  • Yeasts / genetics
  • Yeasts / metabolism


  • Cytochrome b Group
  • Electron Transport Complex III

Associated data

  • PDB/1BCC
  • PDB/1BE3
  • PDB/1BGY
  • PDB/3BCC